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Prognostic outcomes of multifocal bilateral Wilms tumor: A Chinese multicenter cohort study
Jing-Hao Yan1, Xiao-Feng Chang2, Kui-Ran Dong3
1Department of Surgical Oncology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing 100045, China; Children's Hospital of Xinjiang Uygur Autonomous Region Xinjiang Hospital of Beijing Children's Hospita, Urumqi 830000, China.
Background:
Synchronous multifocal bilateral Wilms tumor (mBWT) is rare. This study aimed to evaluate long-term outcomes in children with mBWT and to identify treatment-related risk factors for renal impairment.
Methods:
Patients with synchronous mBWT were identified from a multicenter retrospective cohort. Multifocal disease was defined radiologically as the presence of more than two discrete nodules, each measuring >1 cm in diameter, in one or both kidneys before neoadjuvant chemotherapy (NAC). Survival outcomes and long-term renal function were assessed. Renal impairment was defined as an estimated glomerular filtration rate <90 mL/min/1.73 m2.
Results:
Fifty-six patients with mBWT were included. Ten patients (17.8%) underwent one-step bilateral surgery and (82.2%) underwent staged procedures. Overall, nephron-sparing surgery (NSS) was performed on 100 of 112 renal units (89.2%). After a median follow-up of 6 years, the 5-year overall survival (OS) and event-free survival (EFS) rates were 86.8% and 77.1%, respectively. No significant differences in OS or EFS were observed between patients treated with bilateral NSS and those treated with NSS + total nephrectomy (TN). Renal impairment occurred in 18 patients (32.1%), with 17 developing new-onset or progressive dysfunction during follow-up. Multivariable analysis identified one-step bilateral surgery and collecting system involvement requiring reconstruction as independent risk factors for renal impairment.
Conclusion:
In patients with synchronous mBWT, multifocality alone should not be considered an indication for TN. Staged bilateral NSS appears to be associated with less renal impairment than one-step bilateral surgery or TN + NSS.
